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October 14, 2017NeurologyOpen Access

Quantitative muscle MRI mean fat fraction of the total leg musculature correlated highly with the motor function measure (correlation coefficient -0.845), demonstrating its utility as a biomarker.

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Why the study?

Does quantitative muscle MRI correlate with clinical outcome measures in patients with FSHD?

Population

140 patients with facioscapulohumeral muscular dystrophy comprising the entire clinical spectrum

Design

Cross-sectional

Key result

Quantitative muscle MRI mean fat fraction of the total leg musculature correlated highly with the motor function measure (correlation coefficient -0.845), demonstrating its utility as a biomarker.

Authors

KMKarlien MulSVSanne C. C. VincentenNVNicol C. Voermans

Discussion

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Overview

May enable noninvasive motor function monitoring; leaves open validation in larger trials before clinical adoption.

Study Design

Type

Cross-Sectional (n=140)

Multicenter

No

Structured PICO

Does quantitative muscle MRI correlate with clinical outcome measures in patients with FSHD?

P
Population
140 adults with genetically confirmed facioscapulohumeral muscular dystrophy (FSHD) or asymptomatic gene carriers, aged 18 to 81 years, underwent quantitative muscle MRI and clinical evaluation.
E
Exposure
Quantitative MRI scans of leg muscles
O
Outcome
Correlation of fatty infiltration and TIRM hyperintensities to multiple clinical outcome measures (motor function measure, FSHD clinical score, Ricci score, 6-minute walking test, muscle strength)surrogate

Main Result

Effect estimate: CC -0.845

Quantitative leg muscle MRI is a promising biomarker that captures disease severity and motor functioning in FSHD, detecting pathology before clinical involvement.

Limitations

  • Single-center, single-evaluator design decreases external validity
  • Age at disease onset was collected retrospectively, so results regarding disease duration should be interpreted with caution

Cite This Study

Mul et al. (2017) conducted a cross-sectional in Facioscapulohumeral muscular dystrophy (FSHD) (n=140). Quantitative muscle MRI was evaluated on Correlation between mean fat fraction of total leg musculature and motor function measure (MFM) (CC -0.845). Quantitative muscle MRI mean fat fraction of the total leg musculature correlated highly with the motor function measure (correlation coefficient -0.845), demonstrating its utility as a biomarker.

synapsesocial.com/papers/6a759fd4f1e99ff85ab200aahttps://doi.org/10.1212/wnl.0000000000004647
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Also Consider

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  1. 1Muscle Quantitative MR Imaging and Clustering Analysis in Patients with Facioscapulohumeral Muscular Dystrophy Type 12015 · 56 citations
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  5. 5Quantitative muscle analysis in facioscapulohumeral muscular dystrophy using<scp>whole‐body fat‐referenced MRI</scp>: Protocol development, multicenter feasibility, and repeatability2022 · 21 citations